Finally, in this article we have shown that inhibition of P2X7 through the antagonist A-804598 in SOD1-G93A mice suppresses SQSTM1/p62 up-regulation in lumbar spinal cord, thus confirming P2X7 as an in vivo modulator of the ALS pathological mechanism of autophagy, although further experiments might contribute to identify the cell phenotypes that are more responsible for altered autophagy. Here, SQSTM1 is linked to amyotrophic lateral sclerosis.